IVIg treatment increases thrombin activation of platelets and thrombin generation in paediatric patients with immune thrombocytopenia.

Clinical manifestations and laboratory parameters of haemostasis were investigated in 23 children with newly diagnosed immune thrombocytopenia (ITP) before and after intravenous immunoglobulin (IVIg) treatment. ITP patients with platelet counts of less than 20 × 10 /L and mild bleeding symptoms, graded by a standardized bleeding score (BS), were compared with healthy children with normal platelet counts and children with chemotherapy-related thrombocytopenia. Markers of platelet activation and platelet apoptosis in the absence and presence of platelet activators were analysed by flow cytometry; thrombin generation in plasma was determined.

Pediatric oncologists' perspectives on the use of complementary medicine in pediatric cancer patients in Switzerland: A national survey-based cross-sectional study.

Background: There is a widespread use of complementary therapies among pediatric cancer patients. Previous studies provided evidence that communication between pediatric oncologists (POs) and patients/families about the use of these therapies is often incomplete. Furthermore, nationwide studies on this topic are rare.

The role of time-varying viral shedding in modelling environmental surveillance for public health: revisiting the 2013 poliovirus outbreak in Israel.

Environmental pathogen surveillance is a sensitive tool that can detect early-stage outbreaks, and it is being used to track poliovirus and other pathogens. However, interpretation of longitudinal environmental surveillance signals is difficult because the relationship between infection incidence and viral load in wastewater depends on time-varying shedding intensity. We developed a mathematical model of time-varying poliovirus shedding intensity consistent with expert opinion across a range of immunization states.

Myoepithelial Carcinoma of Soft Tissue With an Gene Fusion in an Infant.

Overall, neonatal cancer is uncommon. Because of its rarity and heterogeneity, diagnosis can be challenging. We report a unique case of a myoepithelial carcinoma in a 7 week old girl.

Local Stage Dependent Necessity of Radiation Therapy in Rhabdoid Tumors of the Kidney (RTK).

Purpose: Rhabdoid tumor of the kidney (RTK) is one of the most aggressive childhood renal tumors. Overall survival ranges from 22% to 47%. The indication for radiation therapy (RT) in usually very young patients is an ongoing discussion.

Intussusception in children: lessons learned from intestinal lymphoma as a rare lead-point.

Background: Most cases of intussusception in children are idiopathic. Rarely, a malignant disease such as intestinal lymphoma may cause intussusception. Due to dramatic changes of expected outcome with chemotherapy regime alone, the surgical management of patients with intestinal lymphoma presenting with intussusception has to be reevaluated.

Acellular dermal matrix allograft versus autogenous connective tissue grafts for thickening soft tissue and covering multiple gingival recessions: a 5-year preference clinical study.

Objectives: The present preference clinical trial compared the long-term outcome of acellular dermal matrix allograft (ADMA) versus autogenous connective tissue graft (CTG) in the treatment of gingival recessions.

Method And Materials: Thirty-nine consecutive patients with 233 Miller Class I and II recessions were treated by one operator (MS) with coronally advanced flaps and in addition either ADMA or CTG harvested from their palate. Clinical parameters were measured by an independent and masked assessor at baseline, 6 months, and 5 years.

Assessing the sensitivity of the polio environmental surveillance system.

Background: The polio environmental surveillance (ES) system has been an incredible tool for advancing polio eradication efforts because of its ability to highlight the spatial and temporal extent of poliovirus circulation. While ES often outperforms, or is more sensitive than AFP surveillance, the sensitivity of the ES system has not been well characterized. Fundamental uncertainty of ES site sensitivity makes it difficult to interpret results from ES, particularly negative results.

Denosumab as a Treatment Alternative for Central Giant Cell Granuloma: A Long-Term Retrospective Cohort Study.

Purpose: Giant cell granuloma (GCG) of the jaw is a rare disease with high morbidity. Various treatment options have been discussed in the past. Since 2010, a pharmaceutical therapy with denosumab seems to have been successful for giant cell tumors of the femur.

Spatial model for risk prediction and sub-national prioritization to aid poliovirus eradication in Pakistan.

Background: Pakistan is one of only three countries where poliovirus circulation remains endemic. For the Pakistan Polio Eradication Program, identifying high risk districts is essential to target interventions and allocate limited resources.

Methods: Using a hierarchical Bayesian framework we developed a spatial Poisson hurdle model to jointly model the probability of one or more paralytic polio cases, and the number of cases that would be detected in the event of an outbreak.

Evaluating cessation of the type 2 oral polio vaccine by modeling pre- and post-cessation detection rates.

The globally synchronized removal of the attenuated Sabin type 2 strain from the oral polio vaccine (OPV) in April 2016 marked a major change in polio vaccination policy. This change will provide a significant reduction in the burden of vaccine-associated paralytic polio (VAPP), but may increase the risk of circulating vaccine-derived poliovirus (cVDPV2) outbreaks during the transition period. This risk can be monitored by tracking the disappearance of Sabin-like type 2 (SL2) using data from the polio surveillance system.

Long-Term Outcome of Catheter-Related Arterial Thrombosis in Infants with Congenital Heart Disease.

Objectives: To investigate the long-term outcome of catheter-related arterial thrombosis in children.

Study Design: Data from clinical and radiologic long-term follow-up of infants with congenital heart disease developing arterial thrombosis following femoral catheterization are presented.

Results: Ninety-five infants with radiologically proven arterial thrombosis because of cardiac catheter (n = 52; 55%) or indwelling arterial catheter (n = 43; 45%) were followed for a median time of 23.

Recruitment limitation of long-lived conifers: implications for climate change responses.

Seed availability and suitable microsites for germination are likely to severely constrain the responses of plant species to climate change, especially at and beyond range edges. For example, range shifts may be slow if seed availability is low at range edges due to low parent-tree abundance or reduced fecundity. Even when seeds are available, climatic and biotic factors may further limit the availability of suitable microsites for recruitment.

Monitoring aspirin therapy in children after interventional cardiac catheterization: laboratory measures, dose response, and clinical outcomes.

Unlabelled: Very few studies have investigated dose response of aspirin and agreement of different platelet function assays in children. One hundred five children were studied at baseline and after interventional cardiac catheterization during aspirin treatment and, in cases of aspirin resistance (AR), after dose increase. Results from arachidonate-induced aggregation (AA) were compared with aggregation induced by ADP, PFA-100 closure times (CTs), urinary 11-dehydro-thromboxane B2 (urinary 11-dhTxB2) levels, and Impact-R % surface coverage.

Port-a-cath-related thrombosis and postthrombotic syndrome in pediatric oncology patients.

Objective: To investigate Port-A-Cath (PAC)-related thrombosis and postthrombotic syndrome (PTS) in children with cancer.

Study Design: The study population was a consecutive cohort of children diagnosed with cancer and a PAC implanted at diagnosis. Children were evaluated for the presence of PAC-related thrombosis by magnetic resonance venography and the presence of congenital prothrombotic risk factors and PTS.

Platelet apoptosis in paediatric immune thrombocytopenia is ameliorated by intravenous immunoglobulin.

To evaluate the role of intravenous immunoglobulin (IVIg) in platelet apoptosis in paediatric immune thrombocytopenia, we investigated the platelets of 20 paediatric patients with acute immune thrombocytopenia (ITP), before and after IVIg treatment. Healthy children with platelet counts in the normal range and children with thrombocytopenia due to chemotherapy were enrolled as controls. All ITP patients presented with platelet counts <20 × 10(9) /l and bleeding symptoms.

Incidence and predictors of indwelling arterial catheter-related thrombosis in children.

Background:  Indwelling arterial catheters (IACs) are used for monitoring and blood sampling purposes in intensive care units. Very limited information is available on the incidence and risk factors of IAC-related thrombosis in children.

Objective:  To investigate the incidence and predictors of IAC-related thrombosis in a tertiary care pediatric hospital.

Use of human protein C concentrates in the treatment of patients with severe congenital protein C deficiency.

Protein C is one of the major inhibitors of the coagulation system that downregulate thrombin generation. Severe congenital protein C deficiency leads to a hypercoagulability state that usually presents at birth with purpura fulminans and/or severe venous and arterial thrombosis. Recurrent thrombotic events are commonly seen.

Self-monitoring of oral anticoagulation therapy in children.

This study aimed to investigate the accuracy of home International Normalized Ratio (INR) self-monitoring in pediatric patients on long-term oral anticoagulation therapy. Statistical and clinical agreement of INR values from capillary whole blood samples measured by 2 different portable prothrombin time monitors (CoaguChek S and XS) and venous blood samples measured by a laboratory coagulation analyzer were evaluated using the Bland-Altman analysis. Eighty-three INR comparisons (56 using the CoaguChek S and 27 using the CoaguChek XS) were obtained from 35 children aged 4 months to 18 years.

Refinement of the critical region for MCKD1 by detection of transcontinental haplotype sharing.

Background: Autosomal-dominant medullary cystic kidney disease type 1 (MCKD1) [OMIM 174000] is a hereditary nephropathy that leads to renal salt wasting and end-stage renal failure at a median age of 62 years. In a Welsh MCKD1 kindred we have recently demonstrated linkage to the MCKD1 locus on chromosome 1q23.1 and refined the critical MCKD1 region to <3.

Familial juvenile hyperuricemic nephropathy and autosomal dominant medullary cystic kidney disease type 2: two facets of the same disease?

Familial juvenile hyperuricemic nephropathy (FJHN) is an autosomal dominant disorder heralded by hyperuricemia during childhood; it is characterized by chronic interstitial nephritis, with marked thickening of tubular basement membranes, and leads to progressive renal failure during adulthood. A gene for FJHN in two Czech families was recently mapped to chromosome 16p11.2, close to the MCKD2 locus, which is responsible for a variant of autosomal dominant medullary cystic kidney disease observed in an Italian family.

Refinement of the gene locus for autosomal dominant medullary cystic kidney disease type 1 (MCKD1) and construction of a physical and partial transcriptional map of the region.

Autosomal dominant medullary cystic kidney disease (MCKD) is an adult onset tubulointerstitial nephropathy that leads to salt wasting and end-stage renal failure. A gene locus (MCKD1) has been mapped on chromosome 1q21. Here we report on a large MCKD1 family of British origin linked to the MCKD1 locus.